Assessment and Management of Patients With Hematologic Disorders

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Copyright © 2008 Lippincott Williams & Wilkins.

Assessment and Management of Patients

With Hematologic Disorders

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Hematologic System• The blood and the blood forming sites, including the

bone marrow and the reticuloendothelial system

• Blood

– Plasma (fluid portion of blood -55%)

– Blood cells

• Hematopoiesis

– The body replenishes its supply of blood cells

– Primary site is bone marrow

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Blood Cells • Erythrocyte: RBC• Leukocyte: WBC

– Neutrophil

– Monocyte

– Eosinophil

– Basophil

– Lymphocyte: T lymphocyte and B lymphocyte

• Thrombocyte: platelet

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Hematologic Studies

• CBC

• Peripheral smear

• Bone marrow aspiration and biopsy

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Blood Smear

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Hemostasis

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Polycythemia• Increased volume of erythrocytes (HCT >55%M/>50%F)• Primary

– Proliferative disorder - bone marrow is hypercellular

– Patients at increased risk for thromboses and bleeding (due to platelet dysfunction)

– Managed usually via phlebotomy

• Secondary– Caused by excessive prodcution of erythropoietin

• May be d/t hypoxic stimulus, neoplasms (epo produced by malignancy)

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Polycythemia• Manifestations

– Hypervolemia and hyperviscosity– Ruddy complexion– Hypertension– Headache– Dizziness, tinnitus– Signs of embolization

• Management– Phlebotomy - reduce HCT to 45% or less– Hydration– Avoid iron supplementation

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Neutropenia• Absolute neutrophil count

– WBC x %neutrophils– <1500/ ul (severe if <500/ul)

• Predisposed to infection– Neutropenia may mask clasic signs of infection - e.g.,

even low grade fever may be of great significance– May progress rapidly to sepsis

• Nursing and collaborative care– Determine cause– Identify offending organism– Institute prophylactic or therapeutic antibiotic– Administer hematopoetic growth factors– Institute protective environmental measures– See table 31-22 and 31-23

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Leukemia • Hematopoietic malignancy with unregulated

proliferation of leukocytes

• Affect blood and blood-forming tissues of bone marrow, lymph system, and spleen

• Types (see table 31-24)

– Acute myeloid leukemia

– Chronic myeloid leukemia

– Acute lymphocytic leukemia

– Chronic lymphocytic leukemia

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Acute Myeloid Leukemia (AML)• Defect in the stem cells that differentiate into all

myeloid cells: monocytes, granulocytes, erythrocytes, and platelets

• Most common leukemia in adults• Affects all ages with peak incidence at age 60• Abrupt onset• Manifestations: fever and infection, weakness and

fatigue, bleeding tendencies, pain from enlarged liver or spleen, hyperplasia of gums, and bone pain

• Treatment is aggressive chemotherapy: induction therapy, BMT, and PBSCT

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Chronic Myeloid Leukemia (CML)• Mutation in myeloid stem cell with uncontrolled

proliferation of cells: excessive neoplastic granulocyts

• Stages: chronic phase, blast crisis

• Uncommon in people under 20; incidence increases with age; mean age is 55 to 60 years

• Life expectancy is 3 to 5 years

• Manifestations (initially may be asymptomatic): malaise; anorexia; weight loss; sternal pain; enlarged, tender spleen; enlarged liver

• Treatment: chemotherapy, BMT, and PBSCT, radiation

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Acute Lymphocytic Leukemia• Uncontrolled proliferation of immature cells from

lymphoid stem cell

• Most common in young children

• Prognosis is good for children; 80% event-free after 5 years, but survival drops with increased age

• Manifestations: fever, bleeding, anemia, mouth sores, symptoms of meningeal involvement and liver, spleen, and bone marrow pain

• Treatment: chemotherapy, BMT or PBSCT

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Chronic Lymphocytic Leukemia• Malignant B lymphocytes, most of which are

mature, may escape apoptosis, resulting in excessive accumulation of cells

• Most common form of chronic leukemia in adults

• More common in older adults and affects men more often

• Survival varies from 2 to 14 years depending upon stage

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Chronic Lymphocytic Leukemia (cont.)

• Manifestations: lymphadenopathy, fatigue, anorexia, hepatomegaly, splenomegaly; in later stages, anemias and thrombocytopenia;; “B symptoms” include fever, night sweats, and weight loss

• Treatment: early stage may require no treatment, chemotherapy,radiation

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Chemotherapy• Induction therapy

– Nursing implications

• Intensification therapy

• Consolidation therapy

• Maintenance therapy

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Nursing Process—Assessment of the Patient With Leukemia

• Health history

• Assess for symptoms of leukemia and complications of anemia, infection, and bleeding – Weakness and fatigue – See table 31-27

• Laboratory tests– Leukocyte count, ANC, hematocrit, platelets,

electrolytes, and cultures reports

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Nursing Process—Diagnosis of the

Patient With Leukemia• Risk for bleeding• Risk for impaired skin integrity• Impaired gas exchange• Impaired mucous membrane• Imbalanced nutrition• Acute pain• Fatigue and activity intolerance• Risk for infection

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Patient With Leukemia (cont.)• Risk for excess fluid volume• Diarrhea• Risk for deficient fluid volume• Self-care deficit• Anxiety• Disturbed body image• Potential for spiritual distress• Grieving diagnoses• Deficient knowledge

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Collaborative Problems/Potential Complications• Infection• Bleeding• Renal dysfunction• Tumor lysis

syndrome• Nutritional depletion• Mucositis• Depression

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Nursing Process—Planning the Care of the

Patient With Leukemia

• Major goals include absence of complications, attainment and maintenance of adequate nutrition, activity tolerance, ability for self-care and to cope with the diagnosis and prognosis, positive body image, and an understanding of the disease process and its treatment

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Interventions• Reduce risk of infection• Reduce risk of bleeding• Mucositis

– Frequent, gentle oral hygiene

– Soft toothbrush, or if counts are low, sponge-tipped applicators

– Rinse only with NS, NS and baking soda, or prescribed solutions

– Perineal and rectal care

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Improving Nutrition• Provide oral care before and after meals

• Administer analgesics before meals

• Provide appropriate treatment of nausea

• Provide small, frequent feedings with soft foods that are moderate in temperature

• Provide a low-microbial diet

• Provide nutritional supplements

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Lymphoma• Neoplasm originating in the bone

marrow and lymphatic structures resulting in the proliferation of lymphocytes

• Hodgkin’s lymphoma

• Non-Hodgkin’s lymphoma

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Hodgkin’s Disease• Unicentric origin

• Reed–Sternberg cell

• Suspected viral etiology (EBV); familial pattern; incidence occurs in early 20s and again after age 50

• Excellent cure rate with treatment

• Manifestations: painless lymph node enlargement; pruritus; B symptoms : fever, night sweats, and weight loss

• Treatment is determined by stage of the disease and may include chemotherapy and/or radiation therapy

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Non-Hodgkin's Lymphoma (NHL)

• Lymphoid tissues become infiltrated with malignant cells that spread unpredictably; localized disease is rare

• Incidence increases with age; the average age of onset is 50 to 60

• Prognosis varies with the type of NHL• Diagnosed via lymph node biopsy and imaging

• Treatment is determined by type and stage of disease and may include interferon, chemotherapy, and/or radiation therapy

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Multiple Myeloma• Malignant disease of plasma cells in the bone

marrow with destruction of bone• M protein and Bence-Jones protein• Median survival is 3 to 5 years; there is no cure

• Manifestations: insidious onset; bone pain, osteoporosis, fractures, elevated serum protein hypercalcemia, renal failure, symptoms of anemia, fatigue, weakness, increased serum viscosity, and increased risk for bleeding and infection

• Treatment may include chemotherapy, corticosteroids, stem cell transplant, and biphosphonates

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Multiple Myeloma• Nursing care is relates to bone involvement

and sequelae from bone breakdown– Hypercalcemia– Fractures– Pain– Infection

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Bleeding Disorders

• Thrombocytopenia• Idiopathic thrombocytopenia purpura (ITP)• Hemophilia• Acquired coagulation disorders: liver

disease, anticoagulants, and vitamin K deficiency

• Disseminated intravascular coagulation (DIC)• Bleeding precautions

– See Nursing care plan 31-2, table 31-15

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Hemophilia• Inherited bleeding disorder, results in

deficiency of factor VIII or IX; varying degrees of disease

• Manifested by hemorrhage into various body parts

• Medical management includes FFP transfusion, recombinant clotting factors, desmopressin (DDAVP)

• Nursing management: control bleeding, patient education aimed at prevention of bleeding

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Disseminated Intravascular Coagulation

• Not a disease but a sign of an underlying disorder; bleeding/thrombotic disorder

• Severity is variable; may be life-threatening

• Triggers may include sepsis, trauma, shock, cancer, abruptio placentae, toxins, and allergic reactions, malignancies, liver disease

• Altered hemostasis mechanism causes massive clotting in microcirculation; as clotting factors are consumed and lost, bleeding occurs; symptoms are related to tissue ischemia and bleeding

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Disseminated Intravascular Coagulation

• Manifestations include simultaneous bleeding and thrombosis;

– Pallor, petechiae, purpura, oozing, bleeding, hematomas, cyanosis, tissue necrosis…

• Lab tests - prolonged PT/PTT, elevated d dimer

– See table 31-20

• Treatment: treat underlying cause, correct tissue ischemia, replace fluids and electrolytes, maintain blood pressure, replace coagulation factors, and use heparin

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Heparin Induced Thrombocytopenia

• Complication of heparin therapy– Higher risk with prolonged use

• Hallmark sign is decline in platelet count, usually after 4-14 days (usually drops by 50% or more)

• Patients at risk for bleeding; also for thrombosis (venous or arterial)

• Treatment includes cessation of heparin, institution of alternative anticoagulation; follow platelet count

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• Review Heparin, Low Molecular Weight Heparin, and Warfarin therapies– Pharmacology book

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Nursing Process—Assessment of the Patient With DIC

• Be aware of patients who are at risk for DIC and assess for signs and symptoms of the condition

• Assess for signs and symptoms and progression of thrombi and bleeding

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Patient With DIC• Risk for fluid volume deficiency

• Risk for impaired skin integrity

• Risk for imbalanced fluid volume

• Ineffective tissue perfusion

• Death anxiety

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Collaborative Problems/Potential Complications

• Renal failure

• Gangrene

• Pulmonary embolism or hemorrhage

• Acute respiratory distress syndrome

• Stroke

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Nursing Process--Planning the Care of the Patient With DIC

• Major goals include maintenance of hemodynamic status, maintenance of intact skin and oral mucosa, maintenance of fluid balance, maintenance of tissue perfusion, enhanced coping, and absence of complications

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Interventions

• Assessment and interventions should target potential /actual sites of organ damage

• Monitor and assess carefully for bleeding and thrombosis

• Avoid trauma and procedures that increase the risk of bleeding, including activities that increase intracranial pressure

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Blood Transfusion Administration• Review patient history including history of

transfusions and transfusion reactions; note concurrent health problems and obtain baseline assessment and VS

• Perform patient teaching and obtain consent

• Equipment: IV (19-20 gauge or greater for PRBCs), appropriate tubing, and normal saline solution

• Procedure to identify patient and blood product

• Monitoring of patient and VS

• Postprocedure care

• Nursing management of adverse reactions

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Complicationssee table 31-33!

• Febrile nonhemolytic reaction• Acute hemolytic reaction• Allergic reaction (mild or severe)• Circulatory overload• Bacterial contamination• Transfusion-related acute lung injury• Delayed hemolytic reaction• Disease acquisition• Complications of long-term transfusion therapy

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Alternative to blood transfusion

• Growth factors

• Erythropoietin

• Granulocyte-Colony Stimulating Factor

• Granulocyte-Macrophage Colony Stimulating Factor

• Thrombopoeitin

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Peripheral Blood Stem Cell Tranplantation and Bone Marrow

Tranplantation• Autologous stem cell transplantation -

uncommon• Donor tranplantation - marrow stem cells or

bone marrow from a donor are transfused– First, patient undergoes intense chemo or radiation

to ablate the patient’s own marrow– Success depends on tissue compatibility and the

patient’s tolerance of the immunosuppression

• Nursing management is focused on prevention of infection and early detection of complication

Documents

Assessment and Management of Patients With Hematologic Disorders